title: Supercomplex assembly determines electron flux in the mitochondrial electron transport chain.
abstract: The textbook description of mitochondrial respiratory complexes (RCs) views them as free-moving entities linked by the mobile carriers coenzyme Q (CoQ) and cytochrome c (cyt c). This model (known as the fluid model) is challenged by the proposal that all RCs except complex II can associate in supercomplexes (SCs). The proposed SCs are the respirasome (complexes I, III, and IV), complexes I and III, and complexes III and IV. The role of SCs is unclear, and their existence is debated. By genetic modulation of interactions between complexes I and III and III and IV, we show that these associations define dedicated CoQ and cyt c pools and that SC assembly is dynamic and organizes electron flux to optimize the use of available substrates.
authors.affiliation: Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.
authors.forename: Esther
authors.initials: E
authors.lastname: Lapuente-Brun
authors.forename: Raquel
authors.initials: R
authors.lastname: Moreno-Loshuertos
authors.forename: Rebeca
authors.initials: R
authors.lastname: Acín-Pérez
authors.forename: Ana
authors.initials: A
authors.lastname: Latorre-Pellicer
authors.forename: Carmen
authors.initials: C
authors.lastname: Colás
authors.forename: Eduardo
authors.initials: E
authors.lastname: Balsa
authors.forename: Ester
authors.initials: E
authors.lastname: Perales-Clemente
authors.forename: Pedro M
authors.initials: PM
authors.lastname: Quirós
authors.forename: Enrique
authors.initials: E
authors.lastname: Calvo
authors.forename: M A
authors.initials: MA
authors.lastname: Rodríguez-Hernández
authors.forename: Plácido
authors.initials: P
authors.lastname: Navas
authors.forename: Raquel
authors.initials: R
authors.lastname: Cruz
authors.forename: Ángel
authors.initials: Á
authors.lastname: Carracedo
authors.forename: Carlos
authors.initials: C
authors.lastname: López-Otín
authors.forename: Acisclo
authors.initials: A
authors.lastname: Pérez-Martos
authors.forename: Patricio
authors.initials: P
authors.lastname: Fernández-Silva
authors.forename: Erika
authors.initials: E
authors.lastname: Fernández-Vizarra
authors.forename: José Antonio
authors.initials: JA
authors.lastname: Enríquez
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D002478:Cells, Cultured; D045304:Cytochromes c / Q000378:metabolism*; D004579:Electron Transport; D042967:Electron Transport Complex I / Q000235:genetics / Q000378:metabolism*; D014450:Electron Transport Complex III / Q000235:genetics / Q000378:metabolism*; D003576:Electron Transport Complex IV / Q000235:genetics / Q000378:metabolism*; D055785:Gene Knockdown Techniques; D057809:HEK293 Cells; D006801:Humans; D051379:Mice; D008810:Mice, Inbred C57BL; D008928:Mitochondria / Q000201:enzymology*; D008969:Molecular Sequence Data; D014451:Ubiquinone / Q000378:metabolism*
chemical_list: D014451:Ubiquinone; D045304:Cytochromes c; C514296:Cox7a2 protein, mouse; D003576:Electron Transport Complex IV; D042967:Electron Transport Complex I; D014450:Electron Transport Complex III